Subacute sclerosing panencephalitis
disease diseaseOn this page
Also known as Dawson diseaseDawson encephalitisImmunosuppressive measles encephalitisSSPESubacute inclusion body encephalitisSubacute sclerosing panencephalitis (disorder) [ambiguous]Van Bogaert diseaseVan Bogaert encephalitis
Summary
Subacute sclerosing panencephalitis (MONDO:0009835) is a disease and 1 clinical trial. A subtype of viral encephalitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: <1 / 1 000 000 (United States) [Orphanet-validated]
- Phenotypes (HPO): 36
- Clinical trials: 1
Clinical features
Epidemiology
Prevalence records
3 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.003 | United States | Validated |
| Annual incidence | 1-9 / 100 000 | 2 | India | Validated |
| Point prevalence | <1 / 1 000 000 | Europe | Not yet validated |
Signs & symptoms
Clinical features (HPO)
36 HPO clinical features (Orphanet curated; top 36 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0000708 | Atypical behavior | Very frequent (80-99%) |
| HP:0001268 | Mental deterioration | Very frequent (80-99%) |
| HP:0001336 | Myoclonus | Very frequent (80-99%) |
| HP:0000716 | Depression | Frequent (30-79%) |
| HP:0000726 | Dementia | Frequent (30-79%) |
| HP:0000737 | Irritability | Frequent (30-79%) |
| HP:0000738 | Hallucinations | Frequent (30-79%) |
| HP:0000746 | Delusion | Frequent (30-79%) |
| HP:0001250 | Seizure | Frequent (30-79%) |
| HP:0001254 | Lethargy | Frequent (30-79%) |
| HP:0001288 | Gait disturbance | Frequent (30-79%) |
| HP:0001332 | Dystonia | Frequent (30-79%) |
| HP:0002360 | Sleep abnormality | Frequent (30-79%) |
| HP:0002371 | Loss of speech | Frequent (30-79%) |
| HP:0010856 | EEG with periodic complexes | Frequent (30-79%) |
| HP:0012332 | Abnormal autonomic nervous system physiology | Frequent (30-79%) |
| HP:0012424 | Chorioretinitis | Frequent (30-79%) |
| HP:0012444 | Brain atrophy | Frequent (30-79%) |
| HP:0012672 | Akinetic mutism | Frequent (30-79%) |
| HP:0030891 | Periventricular white matter hyperintensities | Frequent (30-79%) |
| HP:0031358 | Vegetative state | Frequent (30-79%) |
| HP:0033044 | Motor regression | Frequent (30-79%) |
| HP:0430088 | Anti-measles antibody positivity | Frequent (30-79%) |
| HP:0000572 | Visual loss | Occasional (5-29%) |
| HP:0000573 | Retinal hemorrhage | Occasional (5-29%) |
| HP:0001085 | Papilledema | Occasional (5-29%) |
| HP:0001251 | Ataxia | Occasional (5-29%) |
| HP:0001257 | Spasticity | Occasional (5-29%) |
| HP:0002071 | Abnormality of extrapyramidal motor function | Occasional (5-29%) |
| HP:0002119 | Ventriculomegaly | Occasional (5-29%) |
| HP:0012229 | CSF pleocytosis | Occasional (5-29%) |
| HP:0012708 | Reduced brain N-acetyl aspartate level by MRS | Occasional (5-29%) |
| HP:0012709 | Abnormal brain choline/creatine ratio by MRS | Occasional (5-29%) |
| HP:0100660 | Dyskinesia | Occasional (5-29%) |
| HP:6000397 | CSF oligoclonal immunoglobulin G bands | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | subacute sclerosing panencephalitis |
| Mondo ID | MONDO:0009835 |
| EFO | EFO:0007502 |
| MeSH | D013344 |
| OMIM | 260470 |
| Orphanet | 2806 |
| DOID | DOID:8970 |
| ICD-10-CM | A81.1 |
| ICD-11 | 1098683540 |
| NCIT | C85171 |
| UMLS | C0038522 |
| MedGen | 52527 |
| GARD | 0007708 |
| NORD | 1743 |
| Is cancer (heuristic) | no |
Also known as: Dawson disease · Dawson encephalitis · Immunosuppressive measles encephalitis · SSPE · Subacute inclusion body encephalitis · Subacute sclerosing panencephalitis · subacute sclerosing panencephalitis · Subacute sclerosing panencephalitis (disorder) [ambiguous] · Van Bogaert disease · Van Bogaert encephalitis
Data availability: 12 cell lines.
Disease family
This is a subtype of viral encephalitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › nervous system disorder › central nervous system disorder › encephalomyelitis › encephalitis › infectious encephalitis › viral encephalitis › subacute sclerosing panencephalitis
Related subtypes (11): Powassan encephalitis, acute necrotizing encephalitis, acute hemorrhagic encephalitis, Colorado tick fever, eastern equine encephalitis, herpes simplex encephalitis, tick-borne encephalitis, encephalitis lethargica, mosquito-borne viral encephalitis, HHV-6 encephalitis, borna virus encephalitis
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 1.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT05152771 | Not specified | UNKNOWN | Role of Ketogenic Diet in SSPE |
Related Atlas pages
No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.